Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children2210.24287/1726-1708-2018-17-1-16-22СтатьиDiagnostics of inherited functional platelet disorders (IFPD) in children: correlations between results of flow cytometry, clinical phenotype and results of light transmission aggregometryДиагностика тромбоцитопатий у детей: корреляции исследования функциональной активности тромбоцитов с клинической картиной и результатами агрегометрииhttps://orcid.org/0000-0003-4567-1871FedorovaD. V.ФедороваД.В.
Russian Federation

MD, hemotologist of Outpatient Consultative Unit

Russia 117997, Moscow, Samory Mashela st., 1+7 (495) 287-6570, ext. 1702

daria.fedorova@fcchomoscow.ruhttps://orcid.org/0000-0003-4384-6754ZharkovP. A.ЖарковП.А.
Russian Federation
https://orcid.org/0000-0002-5217-3937IgnatovaA. A.ИгнатоваA.А.
Russian Federation
FedotovA. Yu.ФедотовА.Ю.
Russian Federation
PolokhovD. M.ПолоховД.М.
Russian Federation
https://orcid.org/0000-0001-5209-2099PoletaevA. V.ПолетаевА.В.
Russian Federation
SereginaE. A.СерегинаЕ.А.
Russian Federation
PshonkinA. V.ПшонкинА.В.
Russian Federation
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииDmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Department of Hematology, Oncology and radiotherapy Russian National Research Medical University named after N.I. PirogovФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России09022018171162209082018Copyright ©; 2018, «D. Rogachev NMRCPHOI»Copyright ©; 2018, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2018«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/22

Inherited functional platelet disorders (IFPD) are very heterogeneous group of hemorrhagic diseases. Light transmission aggregometry (LTA) and flow cytometry of platelets are used for diagnostics of IFPD. Flow cytometry is the newer method with some advantages. Our aim was to access correlation between results of LTA and platelet flow cytometry, as well as relationship between results of flow cytometry and clinical phenotypes. Fifty children with suspicion of IFPD were included in initial retrospective analysis. Patients with thrombocytopenia were excluded. Severity of clinical phenotype was accessed by means of ISTH BAT score. Seven patients were diagnosed as having Glanzmann thrombasthenia (GT). Deficiency of δ- and/or α-granules was discovered in 13 children, deficiency of active form of integrin αIIbβ3 in 5, and its combination in 4. None of patients with granules deficiency had pathological findings in LTA results. ISTH BAT score was significantly higher in children with GT in comparison with other patients. But difference in ISTH BAT score was absent between groups with other defects and without any pathological findings in laboratory tests. Our results correspond with previously published data on low sensivity of LTA towards platelet granule disorders. Use of ISTH BAT score was unable to distinguish patients with functional platelet defects and those without any defects accordingly flow cytometry results.

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